Solitary Fibrous Tumours of the Orbit – Clinical Pathology, Therapy and Prognosis

Abstract

Background Solitary fibrous tumours are rare. The aim of this study is to describe the clinical features, therapy and outcome of affected patients and to identify factors associated with recurrence.

Methods Retrospective study of a cohort of 20 patients who underwent surgery for orbital solitary fibrous tumour at the University Department of Oral and Maxillofacial Surgery between 2002 and 2023. Demographic, clinical, and therapeutic data as well as tumour follow-up results were collected. Tumour volume and molecular genetic mutations were retrospectively determined.

Results The median patient age was 49.5 years at initial surgery. The left orbit was affected in 65% of cases. The most common clinical symptom was proptosis (80%). This was reported with a mean lateral difference of 3.9 mm (range: 1 – 10 mm). The tumours were localised predominantly in the intra- and extraconal space, craniolateral quadrant and middle third. The median tumour volume was 7.66 cm³ (range 2.15 – 12.57 cm³). In all patients, the diagnosis was made by pathological examination. All tumours investigated showed a NAB2-STAT6 mutation. The most frequently detected mutation was the fusion NAB2 exon 4 – STAT6 exon 2. All patients were initially managed with frontolateral orbitotomy. Incomplete resection (R1-status) occurred in 35% (n = 7). The recurrence rate was 25% (n = 5), with a median disease-free interval of 45.5 months (range 23 – 130). 80% (n = 4) of recurrences were initially R1-resected.

Conclusion Orbital solitary fibrous tumours are rare tumours and are clinically manifested by signs of displacement of orbital structures. Diagnosis is made by histology and immunohistochemistry and can be proven with the molecular genetic detection of the NAB2-STAT6 mutation. The therapy of choice is complete surgical resection. R1-resection is more likely in the intraconal location as well as in location in the posterior third of the orbit – due to difficult surgical accessibility. The greatest risk factor for the development of recurrence is incomplete surgical excision. Late recurrences are possible, which is why a long-term connection to a specialised clinic is necessary.

Fazit

• Der solitär fibröse Tumor ist eine seltene Tumorentität, der auch in der Orbita vorkommen kann. In den meisten Fällen zeigt er einen benignen Verlauf.

• Der therapeutische Goldstandard ist die vollständige operative Entfernung des Tumors. R1-Resektionen sind in der Orbita aufgrund der erschwerten operativen Zugänglichkeit häufiger als in anderen Lokalisationen.

• Nach einer R1-Resektion ist das Rezidivrisiko erhöht. Risikofaktoren für eine R1-Resektion sind die Lokalisation des Tumors in dem Intrakonalraum und/oder im hinteren Orbitadrittel.

Conclusion

• Solitary fibrous tumors are a rare tumor entity that can also occur in the orbit. In most cases, the course is benign.

• The therapeutic gold standard is complete surgical removal of the tumor. R1 resections are more common in the orbit than in other localizations due to impaired surgical accessibility.

• After R1 resection, the risk of recurrence is increased. Risk factors for R1 resection are localization of the tumor in the intraconal space and/or in the posterior third of the orbit.

Key words

solitary fibrous tumours - orbital neoplasia - frontolateral orbitotomy - retrospective study - nab2-stat6 mutation - recurrence risk factors

Publication History

Received: 05 July 2023

Accepted: 30 August 2023

Article published online:
15 November 2023

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